Treatment of AL amyloidosis without myeloma

AL amyloidosis is a serious complication of monoclonal gammopathy. The therapeutic strategy in amyloidosis associated with myeloma is to decrease the amyloidogenic precursor synthetised by the monoclonal plasmocytic proliferation. However, when systemic amyloidosis complicates a so called "benign" monoclonal gammopathy, this therapeutic approach is debatable. We report 10 cases of AL amyloidosis without myeloma treated by chemotherapy. Eight patients were initially given alkylating agents (cyclophosphamide or melphalan) which had no effect on the clinical progression of their systemic amyloidosis or on the plasma concentrations of the precursor. A limited open clinical trial including 4 patients was then undertaken based on the Vincristine, Adriamycine, Dexamethasone combination recently proposed for cases of resistant myeloma. A 50% reduction in the serum monoclonal protein was observed in 2 patients with this treatment. However, the mean survival of the 10 patients (25 months) was not longer than that previously reported for patients receiving more conventional treatment. The results of this limited trial indicate the need for further controlled therapeutic trials with larger numbers of patients in order to assess the effect of polychemotherapy in patients with AL amyloidosis.